Dermatofibrosarcoma protuberans of scalp with cervical lymph node metastasis

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow growing and locally aggressive tumor of the skin with a high rate of recurrence even after supposedly wide excision. The reports of regional lymph node metastasis and distant metastasis are very rare. Because of the extreme rarity of these cases with metastasis, the experience with management of such patients is very limited. A case of recurrent DFSP of scalp, with metastasis to the regional lymph nodes, in a 17-year-old boy is reported here. This is the second case of DFSP involving scalp and 16th case of DFSP of all sites metastasizing to the regional lymph nodes reported in literature. The patient was treated with wide excision of the lesion and ipsilateral radical neck dissection (including excision of overlying involved skin).     

Surgical treatment of dermatofibrosarcoma protuberans. A retrospective study of 20 cases with review of literature

The clinicopathological features and results of surgical treatment of 20 patients with dermatofibrosarcoma protuberans (DFSP) were reviewed. All patients have been observed until the present time or death. The primary treatment usually consisted of marginal, wide or radical excision. Re-excision because of recurrence was performed in six of the 20 patients who were treated primarily only by marginal excision. Following re-excision two patients have remained disease-free until the present, the other four of these six patients had a second recurrence. Three of these four patients with a second relapse were successfully treated by excision. Although six patients out of 20 had 13 recurrences at the site of primary therapy, no patient developed lymph node or distant metastases. To date, none of the remaining 14 patients has had recurrence, all having been treated by excision of from 1 to 5 cm, the mean follow-up time was 8.75 years. Based on our data, the high recurrence rate after marginal surgical treatment implies an initial radical resection.     

Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate.

PURPOSE: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) beta-chain from gene rearrangement as a key pathogenetic factor, now susceptible of molecular-targeted therapy. The aim of this retrospective analysis was to explore the outcome of patients with primary or recurrent DFSP. PATIENTS AND METHODS: Two hundred eighteen patients surgically treated at the Istituto Nazionale per lo studio e la cura dei Tumori (Milan, Italy) over 20 years were reviewed. Local relapse, distant metastasis, and survival were studied. RESULTS: One hundred thirty-six patients (62.4%) presented with a primary DFSP, while 82 patients (37.6%) had a recurrent disease. In the primary group, margins were microscopically positive in 11.8%, while in the recurrent group they were positive in 14.6% (P =.613). In the primary group, patients undergoing re-excision after inadequate previous surgery had residual disease in 62% of cases. Reconstructive surgery was needed in 30%, significantly more frequently in patients with a recurrence or a head and neck tumor. The crude cumulative incidence of local relapses was 4% at 10 years, and 2% at 10 years for distant metastases. No significant difference was found between primary and recurrent patients, as well as between positive and negative margins. CONCLUSION: This being one of the largest mono-institutional series of DFSP, we confirm that long-term outcome is excellent, in terms of both local and distant control, after a wide excision with negative margins. Reconstructive surgery is often needed. Novel medical therapies will be of use in a limited subgroup of patients.     

Merkel cell cancer of the skin.

Merkel cell carcinoma (MCC) is a rare malignant cutaneous tumor of the elderly with rapidly growing skin nodules found predominantly on sun-exposed areas of the body. The vast majority of patients present with localized disease, while up to 30% have regional lymph node metastases. Despite local excision and the incidence of local recurrence, regional lymph node metastases and distant metastases is high and usually occurs within 2 years of primary diagnosis. The optimal treatment for patients with MCC remains unclear. The best outcome is achieved with multidisciplinary management including surgical excision of primary tumor with adequate margins and post-operative radiotherapy (RT) to control local and regional disease. Patients with regional nodal metastases should be treated with lymph node dissection plus RT. Adjuvant chemotherapy (CT) should be considered as part of the initial management. In case of metastatic disease CT based on regimens used for small-cell lung cancer is the standard treatment of care.     

Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor of the skin. Clinically, it often masquerades as a benign, indolent tumor on the trunk and extremities. Microscopically, it extends far beyond assessed clinical margins, spreading locally in the dermis, subcutaneous tissue, and muscle. The local recurrence rate in patients with DFSP who undergo wide local excision ranges from 0% to 21%. Recent preliminary reports indicate more consistently favorable cure rates with Mohs micrographic surgery (MMS). However, to date only a few scattered reports have documented long-term 5-year follow-up. The authors present data on 29 patients with DFSP who underwent MMS. In addition, they reviewed the medical literature to summarize the accumulated experience of MMS treatment in the management of DFSP. METHODS: The authors conducted a retrospective review of a series of 40 consecutive patients with DFSP who underwent MMS over the last 20 years. Of these, there were 29 patients with > 5 years of follow-up who formed the basis of the current review. The literature also was searched for patients with DFSP who underwent MMS with > 5 years of follow-up RESULTS: At the University of Wisconsin Mohs Surgery Clinic, 29 patients with > 5 years of follow-up were treated. There were 16 women and 13 men. Eight patients developed recurrent disease after previous non-Mohs treatment. Site distribution was 45% head and neck and 55% trunk and extremities. In the current series, there were no local recurrences, with a local 5-year cure rate of 100%. In the literature review, which included the current series, there were 136 patients with DFSP who underwent Mohs surgery with > 5 years of follow-up. Nine patients in the current series developed local recurrences, including five patients who underwent a second Mohs procedure. The local cure rates after the first and second Mohs surgeries were 93.4% and 98.5%, respectively. The rate (percent) and time to local recurrence was 50% at 3 years and 75% at 5 years. However, 25% of local recurrences appeared late, after the usual 5-year recommendation. CONCLUSIONS: In a series of 29 patients with of DFSP and in an accompanying update of the medical literature, 136 patients with DFSP underwent MMS with > 5 years of follow-up. There were no regional and/or distant metastases. However, late recurrences beyond the usual recommended 5-year follow-up may occur. Therefore, all patients with DFSP, especially those with recurrent tumors, should be followed for an extended period. The accumulated data continue to confirm that, when DFSP is discovered early and is accessible readily to excision by MMS, a favorable outcome can be expected with minimal trauma or sacrifice of adjacent normal structures and with a low recurrence rate.     

Marjolin's ulcer. The LSU experience

Skin cancers originating in areas of chronic injury (Marjolin's ulcers) are thought to behave in a more aggressive fashion than those due to other causes. The initial surgical treatment, especially the management of the regional lymph nodes, remains controversial. This review of 37 patients with Marjolin's ulcer evaluated the effectiveness of local surgical treatment at our center. Three of 18 patients treated with amputation developed recurrences and died of metastatic disease. Five of 16 patients treated by wide excision developed recurrences, two dying with metastatic disease, the remaining three apparently cured by additional surgery. Only six (17.6%) of the patients treated by "local" modalities, i.e., wide excision or amputation, developed subsequent regional node metastases. Recurrences following local excision tend to be local and can usually be managed by re-excision or amputation. It can be inferred from this study that prophylactic node dissections are not required in most patients with Marjolin's ulcers.     

Time periods of the occurrence of local recurrences and the late results of the treatment of skin melanomas following surgical excision of the primary tumor

An analysis of the clinical data on 175 cases of local recurrence of malignant melanoma of the skin after surgery established the rate of incidence of these lesions in primary patients at 5.4 +/- 0.7%. In 41.7 +/- 3.7%, local lesions co-occurred with the development of regional, intransit and distant metastases. In 88.0 +/- 2.6%, local recurrences appeared within the first three years after excision of primary tumor. The rate of survival of such patients within five years after removal of recurrent tumor was 31.4 +/- 3.9% and 21.2 +/- 4.1%--after ten years.     

Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with a propensity for local recurrence. Treatments with wide excision, Mohs surgery, and other approaches have been reported with widely variable local control rates. The objective of this study was to review the experience with a multidisciplinary approach employing wide excision and Mohs surgery selectively in the treatment of patients with DFSP at a single academic institution over the past 10 years. METHODS: The records of 62 patients with 63 DFSP tumors who underwent wide excision, Mohs surgery, or a multidisciplinary combination approach from January 1991 to December 2000 were reviewed retrospectively. Primary endpoints included the ability to extirpate the DFSP lesion completely, the tumor recurrence rate, and the need for skin grafts or local tissue flaps. Additional objectives included defining surgical practice patterns at the authors' institution. RESULTS: Sixty-three DFSP lesions were removed from 62 patients. At a median follow-up of 4.4 years, no local or distant recurrences were detected in any patient. Forty-three lesions were treated with wide local excision, 11 lesions were treated with Mohs surgery, and 9 lesions were treated with a combination approach. Ninety-five percent of lesions that were approached initially with wide local excision were cleared histologically. Two patients (5%) received postoperative radiation for positive margins after undergoing maximal excision. Eighty-five percent of lesions that were approached initially with Mohs surgery were cleared histologically. The remaining 15% of lesions subsequently were cleared surgically with a wide excision. DFSP lesions that were approached initially with Mohs surgery tended to be smaller. Patients with head and neck lesions most often underwent Mohs surgery or were treated with a multidisciplinary combination approach (87%). CONCLUSIONS: Wide local excision with careful pathologic analysis of margins was found to have a very low recurrence rate and was used for the majority of patients with DFSP lesions at the authors' institution. Wide local excision, Mohs surgery, and a multidisciplinary combination approach, selected based on both tumor and patient factors, were capable of achieving very high local control rates in the treatment of DFSP. The evolution of a multidisciplinary approach has provided a level of expertise that no single individual could achieve for the treatment of the full spectrum of DFSP lesions at the authors' institution.     

Conservative surgical management of superficially invasive cutaneous melanoma

Between 1976 and 1980, 136 patients were evaluated for primary treatment of cutaneous melanoma. Forty-nine lesions were invasive to Clark's Level II (38 patients) or III (11 patients) with a thickness of 0.3 to 1.2 mm. Conservative re-excision was advised as definitive therapy for these patients. The margin of resection was defined as the maximum excision that would allow primary closure of the wound. Pathology reports of the re-excised specimens revealed the narrowest margins to be 0.7 to 4 cm. Unexpected residual tumor was present in 2 specimens and melanocytic hyperplasia in 12 specimens. After a follow-up period of 2.5 to 7.0 years, there have been no local recurrences. One patient developed regional lymph node metastases 16 months and, then central nervous system (CNS) metastases 25 months after primary treatment. A second patient died with pulmonary metastases 4.5 years after initial therapy. Melanomas that are not deeply invasive do not require wide excision and skin grafting for local control. Occasionally these thin lesions do produce systemic metastases, emphasizing the need for long-term follow-up of even "low-risk" patients.     

Merkelzellkarzinom

Merkel cell carcinoma is a rare, highly aggressive skin tumor with neuroendocrine features found in older people. The pathogenesis is associated with immunosuppression, chronic UV light exposure and the Merkel cell polyomavirus. Clinically, Merkel cell carcinoma presents as a solitary, cutaneous or subcutaneous, red to bluish node. Due to early lymphogenic metastasis, locoregional metastases are already present in approximately 30% of cases at the time of diagnosis. The frequent local recurrences as well as the regional and distant metastases usually appear within the first 2–3 years after the initial diagnosis. The first treatment after diagnosis consists of complete surgical removal of the primary tumor with wide safety margins as well as a sentinel lymph node biopsy. Subsequently, adjuvant irradiation of the primary site should be performed. By additional radiotherapy of the regional lymph node stations, the rate of locoregional recurrence can be reduced. For systemic therapy of advanced Merkel cell carcinoma checkpoint inhibitors targeted against the PD-1/PD-L1 axis have proven to be highly and durably effective. In contrast the formerly frequently used chemotherapy shows moderate to good response rates but they are as a rule very short-lived.     

Patterns of recurrence in patients with squamous cell carcinoma of the vulva. A multicenter CTF Study

BACKGROUND: Invasive vulvar carcinoma is a rare disease with an incidence rate of 3-5% of all female genital neoplasms. The current study discusses the limited number of articles in the literature regarding the patterns of recurrence as well as the clinical outcome of patients with recurrent disease based on a consistent and consecutive series of cases. METHODS: A common clinical chart focusing on the study of patterns of recurrence was used in five Italian gynecologic institutions with uniform criteria of surgical nomenclature, pathologic variables, and sites of recurrence. Between 1980-1994, 502 cases of primary invasive squamous carcinoma of the vulva were registered consecutively, treated, and considered for this multicentered study. RESULTS: Of 502 patients, 187 (37.3%) developed a recurrence. Distribution of the recurrences by site was as follows: perineal, 53.4%; inguinal, 18.7%; pelvic, 5.7%; distant, 7.9%; and multiple, 14.2%. In a multivariate analysis, 3 characteristics appeared to be statistically correlated with the risk of recurrence: International Federation of Gynecology and Obstetrics Stage > II (P = 0.029), positive lymph nodes (P = 0.009), and vascular space invasion (P = 0.004). The 5-year survival rate was 60% for perineal recurrences, 27% for inguinal and pelvic recurrences, 15% for distant recurrences, and 14% for multiple recurrences. CONCLUSIONS: In the current study the prognostic factors found to have statistical significance as prognostic factors for risk of recurrence were tumor dimension, lymph node involvement, and stromal and vascular space invasion. The presence of inguinal lymph node metastases was predictive of multiple and distant recurrences with a low rate of incidence of isolated perineal recurrence (27%) compared with negative lymph node cases (57.5%). Survival analysis of recurrent disease showed that the surgical resection of local recurrences may provide acceptable results (51% at 5 years). This observation may justify a follow-up program aimed at identifying those patients with early local recurrence suitable for radical resection.     

Outcomes of surgery for dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma usually presenting as nodular cutaneous mass on the trunk and proximal extremity. The tumour grows slowly, typically over years. The standard treatment is wide local excision with at least a 3-cm margin. The local regional recurrence is up to 50%, emphasizing the need for wide margins for local control. A small fraction of DFSP may metastasize, but on histological examination such tumours have features of fibrosarcomas rather than DFSP. HYPOTHESIS: This study was done to review our experience of the time interval to recurrence of DFSP. DESIGN: A retrospective review was undertaken to identify patients with DFSP in our university teaching hospital. METHODS: All patients received their primary surgical treatment in our department between February 1968 and June 2001. Treatment consisted of wide local excision with margins of at least 3 cm. The chi-square test and Fisher's exact test were performed to determine the relationship between recurrence and clinicopathological variables. We evaluated the prognostic variables using the Kaplan-Meier method with log-rank comparison. RESULTS: The median follow-up period was 59 months. The 5 and 10-year disease-free survival (DFS) were 86 and 76%, respectively. The overall recurrence rate was 16.7%. The mean time to recurrence was 38+/-12 months (range 1-100 months). In 30% of those patients with recurrences, the local regional recurrence was after 5 years. CONCLUSION: Wide local excision with good margins decreases local regional recurrences in patients with DFSP. Close surveillance is necessary even beyond 5 years because late recurrences occur.     

A comprehensive review of the treatment of Merkel cell carcinoma

Merkel cell carcinoma (MCC) is an uncommon but malignant cutaneous neuroendocrine carcinoma with a high incidence of local recurrence, regional lymph node metastases, and subsequent distant metastases. The etiology of MCC remains unknown. It usually occurs in sun-exposed areas in elderly people, many of whom have a history of other synchronous or metachronous sun-associated skin lesions. The outcome for most patients with MCC is generally poor. Surgery is the mainstay of treatment. The role of adjuvant therapy has been debated. However, data from recent development support a multimodality approach, including surgical excision of primary tumor with adequate margins and sentinel lymph node dissection followed by postoperative radiotherapy in most cases, as current choice of practice with better locoregional control and disease-free survival. Patients with regional nodal involvement or advanced disease should undergo nodal dissection followed by adjuvant radiotherapy and, perhaps, systemic platinum-based chemotherapy in most cases.     

Primary Adenoid Cystic Carcinoma of the Skin with Multiple Local Recurrences

Primary adenoid cystic carcinoma (PACC) of the skin is a rare tumor with fewer than 70 cases studied in detail in the English literature. This type of tumor shows a prolonged course and a growth pattern usually manifested by multiple local recurrences and has a low potential for distant metastases. The most important modality for primary treatment is surgical resection followed by radiotherapy. We report a woman aged 43 years at the time of diagnosis, who presented with a slow-growing nodule in the right axilla without lymph node enlargement. A wide local excision was performed, and the histology revealed an adenoid cystic carcinoma. During the next 24 years, another four local recurrences were excised (the last one in 2015) and confirmed histologically to be adenoid cystic carcinoma. The patient was given 44 Gy of radiotherapy after the second surgery in 1996. PACC of the skin is a rare tumor with insufficient data concerning the efficacy of the surgical technique and chemotherapy and radiotherapy treatment, even more so in the case of multiple recurrences. After the last recurrence, the patient was offered an active follow-up based on the long tumor-free intervals in the past and because the site of the primary tumor allowed further surgical excisions in future recurrences.Key Words: Adenoid cystic carcinoma, Cylindroma, Skin     

Prognosis of level V malignant melanoma

Level V melanomas have been reported to have a poor prognosis, but in-depth analyses of prognostic factors and treatment have not been reported. From 1952 through 1982, 41 patients presented with primary Clark's Level V melanomas. There were 23 patients who presented with clinical Stage I disease and 18 with Stage II. Among Stage I patients, 9 were treated by wide excision alone and 13 underwent wide excision plus prophylactic regional lymph node dissection (RLND); 8 of 13 patients had histologically positive nodes. Twelve Stage II patients were treated by wide excision and RLND (including three hemipelvectomies), and four refused surgery. The 5-year survival was 52%. For Stage I patients, survival was 62% and disease-free survival (DFS) 28% at 5 years; 6 of 10 recurrences were local or regional only. Prophylactic RLND reduced the incidence of recurrence but did not appear to influence survival rates. Among 14 evaluable Stage II patients, overall survival was 60% and DFS 42% at 3 years; of 4 patients who subsequently had a recurrence, 3 had distant metastases. All seven patients with distant metastases at the time of first recurrence died of disease within 14 months (median, 4 months) of detection of metastatic disease. Primary melanomas of the foot (11 patients) and trunk (4 patients) appeared to have a worse prognosis than other sites. Ulceration (seen in 21 patients) did not appear to significantly influence outcome. These data suggest that most patients with Level V melanoma present with clinically localized disease. Prophylactic RLND did not significantly affect overall survival. The invasiveness of these deep tumors appears to reduce the influence of other factors, including primary site, sex, race, and ulceration. The prognosis of patients with Level V melanoma, even with clinically or histologically positive lymph nodes, is not hopeless, and these patients should be treated aggressively.     

复发性喉癌患者的临床特点和影响预后因素的分析

喉癌复发是影响预后的重要因素,但对复发性喉癌患者的临床特点和影响预后因素的研究较少。本研究的目的是总结分析复发性喉癌患者的临床特点,探讨影响复发性喉癌患者预后的主要因素。     

Evaluation of modalities for recurrent breast cancer patients

Of 342 breast cancer patients radically operated on in the Second Department of Surgery, School of Medicine, Chiba University during 1965-1988, treatment for 75 recurrent patients were evaluated by the initial modes of recurrence. The modes of recurrence were classified into distant metastases, local lymph node recurrence (axillary, parasternal and supraclavicular nodes) and chest wall recurrence according to the General Rules for Clinical and Pathological Recording of Breast Cancer. Of 75 recurrent patients, distant metastases were seen as common as 77.3%, followed by recurrences of local lymph nodes (14.7%) and chest wall (8.0%). The number of patients in each mode of recurrence increased in relation to increase in the size of tumor and the number of metastatic lymph nodes at the time of the first operation. Histologically, scirrhous carcinoma was most common in chest wall recurrence. 2-year disease-free survival rates of distant metastases, local lymph node recurrence and chest wall recurrence were 44.6%, 24.2% and 16.7%, respectively. 5-year survival of bone metastasis with chemo-endocrine therapy was as significantly favorable as 60%, compared to chemo- or radiotherapy alone (p less than 0.01). However, 5-year survival of lung metastasis with or without endocrine therapy revealed no significant difference. Local lymph node recurrence with the combination of resection, radio- and/or chemotherapy produced a trend toward showing more favorable survival than that without resection.     

胸段食管鳞癌左开胸二野清扫术后复发规律的探讨

目的:分析胸段食管鳞癌根治性左侧开胸二野清扫术后复发的规律,为术后辅助性治疗提供依据。方法:收集1998年6月-2012年12月收治的111例胸段食管鳞癌术后复发的患者,分析其复发情况。结果:111例患者中位复发时间为16.0个月,82.9%患者在术后3年内复发,复发时临床表现以声音嘶哑为最常见（36.0%）。复发类型:单纯局部区域复发76.6%,单纯远处转移6.3%,区域复发合并远处转移17.1%（P=0.000）。局部复发中纵隔淋巴结转移60.4%,下颈锁骨区淋巴结转移48.6%,腹腔淋巴结转移10.8%,吻合口复发15.3%,瘤床区复发1.8%。远处转移26例中,肺转移占50.0%。不同原发部位食管癌之间复发区域的差异无统计学意义（P>0.05）。纵隔淋巴结转移中,上纵隔复发率55.9%,中纵隔19.8%,下纵隔淋巴结转移1.8%（P=0.000）,在纵隔淋巴结复发中,1、2区淋巴结复发率分别为42.3%和34.2%,4区22.5%,7区18.0%。采用logistic分析复发部位与临床资料相关性,结果显示纵隔淋巴结转移与T分期有关。结论:胸段食管鳞癌根治性左开胸二野清扫术后复发多在术后3年内发生,以声音嘶哑常见,淋巴结复发为主要复发类型,其中下颈锁骨区及上纵隔1、2区淋巴结复发多见,不同原发部位食管癌之间复发区域无差异。     

Molecular targeting of dermatofibrosarcoma protuberans: a new approach to a surgical disease

Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignancy of the skin and subcutaneous tissues that only rarely forms distant metastases. More than 90% of cases are associated with a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the platelet-derived growth factor B gene on chromosome 22. Management of this disease is primarily surgical with excellent rates of local control obtained using either wide local excision or Mohs micrographic surgery. Data have recently shown that inhibiting platelet-derived growth factor receptors (PDGFR) with imatinib can induce high rates of clinical response in patients with unresectable or metastatic DFSP. These data have led to approval of imatinib by the U.S. Food and Drug Administration for treating uresectable DFSP. Although wide surgical excision remains standard care, patients with locally advanced disease not suitable for surgical excision can be treated with the PDGFR-inhibitor imatinib, which sometimes allows residual DFSP to be surgically excised.     

Efficacy of intralesional BCG therapy in guinea pigs with disseminated tumor.

It has been previously demonstrated that transplanted syngeneic tumors established in the skin of inbred (strain-2) guinea pigs regressed and regional lymph node metastases were eliminated after intralesional injection of viable Mycobacterium bovis (BCG). During the course of this reaction there is the development of tumor-specific immunity. This experimental model was further manipulated in order that it would more closely approximate a clinical reality. In the present study an evaluation was made of the effectiveness of the developing tumor-specific immunity in this BCG therapy model, to abrogate artifically induced distant tumor deposits and to assess the requirement for tumor-specific immunity in the local BCG-mediated tumor regression. During BCG-mediated regression of established intradermal tumor, the developing tumor-specific immunity inhibited the growth of artificially induced vascular metastases in animals receiving a 10(4) or 10(5) tumor cell dose. However, there is a direct causal relationship between the distant tumor burden and the escape of skin tumor and regional lymph node metastases from BCG-mediated regression. Thus, multiple tumor deposits as low as 10(4) cells are capable of competing for or preempting tumor-specific immune reactivity, which must be a requirement during some phase of the intralesional BCG-mediated therapy of established tumor and regional lymph node metastases. Thus, a significant therapeutic effect could be achieved in guinea pigs with established skin tumors and limited vascular metastases when the modality of therapy included BCG intralesional injection, followed 6 weeks later by surgery of the treated skin tumor and regional lymph node.